PCR testing for herpesviruses in aqueous humor samples from patients with and without clinical corneal endothelial graft rejection.

To compare prevalence of positive PCR tests for herpesviruses between patients with and without a history of clinical corneal endothelial allograft rejection (AGR). Retrospective cross-sectional study with two-group comparison. A total of 307 aqueous humor (AH) samples from 235 Patients and 244 eyes who underwent penetrating keratoplasty or Descemet membrane endothelial keratoplasty or had a diagnostic AH aspiration due to clinical AGR between 2019 and 2023 were tested for DNA of herpes simplex virus (HSV), varicella-zoster virus (VZV), cytomegalovirus (CMV), and Epstein-Barr virus (EBV). PCR test results were compared between the two groups (with/without AGR). Another sub-analysis examined the results of patients without a history of herpetic keratitis. A total of 8% of eyes with clinical AGR (9/108) had a positive PCR result for one of the herpesviruses (HSV:3, CMV:3, EBV:2, VZV:1). All patients in the group without AGR had negative PCR results for all previous viruses (0/136). The difference was statistically significant (p < 0.001). The sub-analysis of eyes without a history of herpetic keratitis also revealed significantly more positive herpes PCR results (7/87) in eyes with AGR than in eyes without AGR (0/42, p = 0.005). Clinical AGR after keratoplasty shows a significant correlation to viral replication. Herpetic infection and AGR could occur simultaneously and act synergistically. Timely differentiation between active herpetic infection and/or AGR is pivotal for proper treatment and graft preservation.

Surgical management of complicated Descemet's membrane detachment in corneas without prior endothelial keratoplasty.

PURPOSE: To provide insights into morphologic and functional features of eyes with complicated Descemet's membrane detachment (DMD) and report clinical outcomes after surgical intervention. METHODS: Retrospective study of 18 eyes with complicated DMD between 2010 and 2022. Complicated DMD was defined if any of the following criteria applied: prior penetrating keratoplasty (PKP), corneal thinning, total DMD or persistent DMD after Air/Gas-Descemetopexy. Causes, surgical management, and clinical outcomes were analyzed. Scheimpflug tomography, anterior segment optical coherence tomography (AS-OCT) and histologic examination were performed to characterize corneas with DMD. RESULTS: Fourteen eyes with prior PKP developed spontaneous DMD after 24.2 ± 12.9 years (range = 18 months - 47 years, median = 25.7 years). Complicated DMD without prior PKP was associated in three eyes after cataract surgery and in one eye after infectious keratitis. In cases with previous PKP, AS-OCT demonstrated rupture of Descemet's membrane (DM) in five eyes and spontaneous reattachment was found in four eyes within 8 weeks of initial diagnosis, with no rupture of DM in any of the cases. There was no rupture of DM in corneas without previous PKP. After prior keratoplasty, definitive surgical treatment was repeat PKP in 13 eyes and Air/Gas-Descemetopexy in one eye. In corneas without prior keratoplasty, three eyes underwent PKP and one eye Air/Gas-Descemetopexy. Histological examination of two corneal explants revealed a severely thinned graft-host junction and a disrupted DM close to the graft-host junction. Visual acuity improved from 1.80 ± 0.58 logMAR to 0.75 ± 0.69 logMAR after prior PKP and from 1.45 ± 0.65 logMAR to 0.85 ± 1.13 logMAR without prior PKP. The postoperative course was uneventful in 16 of 18 eyes. CONCLUSION: PKP is an effective treatment option for complicated DMD, especially in ectatic corneas, whereas Air/Gas-Descemetopexy or Descemet Membrane Endothelial Keratoplasty do not address the primary issue of the curvature anomaly.

[Comparison of the Effect of Autologous Serum on Therapy Resistant Corneal Erosions and Ulcers on the Corneal Graft vs. the Patient's Own Cornea]. / Die Wirkung von autologen Serumaugentropfen bei therapieresistenten Erosiones und Ulcera der Kornea bei eigenem im Vergleich zu transplantiertem Gewebe.

PURPOSE: The aim of this study is to compare the healing of corneal epithelial defects or ulcers on the corneal graft in comparison with the patient's own cornea after treatment with 100%, undiluted autologous serum eye drops. METHODS: In a retrospective study over 7 years, we analysed 263 treatments with autologous serum eye drops of persistent corneal epithelial defects (erosions [88%] vs. ulcers [12%]). We compared the epithelial healing tendency of patients with defects on their own cornea (51.9%) vs. patients who had previously undergone penetrating keratoplasty (48.1%). Complete epithelial healing during the 28 days of treatment was considered as therapeutic success. In addition, the recurrence rate of the epithelial defects after finishing the therapy was analysed. RESULTS: 88.2% of the epithelial defects healed during 28 days of therapy. The recurrence rate during follow-up was 5.1%. There was no significant difference with respect to success rate between corneal defects on the patient's own cornea (87.8%) and on the graft (88.6%; p = 0.137). There was a significantly lower success rate for corneal ulcers (74.2%) than for erosions (90.3%; p < 0.001). The recurrence rate of erosions was 4.4%, vs. 4.3% in ulcers during follow-up. CONCLUSION: The results of our study suggest that autologous serum eye drops are a non-invasive and safe alternative treatment for persistent corneal epithelial defects - with no significant difference in patients with a defect on their own cornea vs. defects on the corneal graft. The success rate, but not the recurrence rate, is significantly worse in ulcers than in erosions.

Reliability analysis of successive Corvis ST® measurements in keratoconus 2 years after accelerated corneal crosslinking compared to untreated keratoconus corneas.

PURPOSE: To assess the reliability of successive Corvis ST® measurements (CST, Oculus, Wetzlar, Germany) in keratoconus (KC) ≥ 2 years after accelerated corneal crosslinking (9 mW/cm2, 10 min, 5.4 J/cm2) compared to untreated KC corneas. METHODS: Three successive CST measurements per eye were performed in ≥ 2 years after CXL (CXLG, n = 20 corneas of 16 patients) and a control group consisting of non-operated, ABC-stage-matched KC corneas according to Belin's ABCD KC grading (controls, n = 20 corneas, 20 patients). Main outcome measures included maximal keratometry (Kmax), the Belin/Ambrósio-Enhanced-Ectasia-Deviation-Index BAD-D; the biomechanical parameters A1 velocity, deformation amplitude (DA) ratio 2 mm, Ambrósio relational thickness to the horizontal profile (ARTh), integrated radius, stiffness parameter A1 (SP-A1), and the Corvis Biomechanical Factor (CBiF, the linearized term of the Corvis Biomechanical Index). Mean values, standard deviations, and Cronbach's alpha (CA) were calculated. RESULTS: Both groups were tomographically comparable (BAD: 11.5 ± 4.7|11.2 ± 3.6, p = 0.682, Kmax: 60.5 ± 7.2|60.7 ± 7.7, p = 0.868 for controls|CXLG, paired t-test). A1 velocity (mean ± SD: 0.176 ± 0.02|0.183 ± 0.02, p = 0.090, CA: 0.960|0.960), DA ratio 2 mm (6.04 ± 1.13|6.14 ± 1.03, p = 0.490, CA: 0.967|0.967), integrated radius (12.08 ± 2.5|12.42 ± 1.9, p = 0.450, CA: 0.976|0.976), and CBiF (4.62 ± 0.6|4.62 ± 0.4, p = 0.830, CA: 0.965|0.965) were also comparable (controls|CXLG). ARTh was significantly higher in controls (177.1 ± 59, CA: 0.993) than after CXL (155.21 ± 65, p = 0.0062, CA: 0.993) and SP-A1 was significantly higher after CXL (59.2 ± 13, CA: 0.912) than in controls (52.2 ± 16, p = 0.0018, CA: 0.912). CONCLUSION: ARTh and SP-A1 differed significantly between controls and CXLG. Biomechanical measurements were generally of excellent reliability in both groups. CXL seems to affect biomechanical measurements of human corneas over more than 2 years.

Baseline Characteristics of 1976 Patients With Ectatic Corneal Disorders at a Single Center From 2010 to 2021: A Cross-Sectional Study of the Homburg Keratoconus Center.

OBJECTIVES: The study objective was to analyze the baseline characteristics of keratoconus (KC) patients at the Homburg Keratoconus Center from 2010 to 2021. METHODS: This cross-sectional study included 3,674 eyes, with analysis of demographics, clinical findings, visual function, endothelial measurements, and topographic, tomographic, and corneal biomechanical data from the first visit. RESULTS: Mean patient age was 36.3±13.8 years. The mean uncorrected distance visual acuity in log of minimal angle of resolution was 0.60 (20/80, Snellen equivalent), and the corrected mean was 0.3 (20/40). Of 1976 patients, 48.9% reported eye rubbing. Mean values (ranges) were 49.4±6.3 (36.3-78.0) D for steep keratometry, 462.4±66.0 (48.0-659.0) µm for thinnest corneal thickness, 9.7±8.7 (-0.5 to 88.8) for Belin/Ambrósio enhanced ectasia total deviation, 0.8±0.4 (0.0-1.0) for the Corvis biomechanical index, 0.9±0.2 (0.0-1.0) for the tomographic biomechanical index, 0.1±0.5 (-0.9 to 2.0) for the KC match index, 8.3±1.8 (2.2-17.7) mm Hg for corneal hysteresis, 7.1±2.2 (0.0-17.0) mm Hg for corneal resistance factor, and 2,562.9±326.3 (1,011-3,937) cells/mm2 for endothelial cell density. The average ABCDE KC stage was A2B3C1D1E2. Distance-corrected visual acuity correlated strongly with topometric, tomographic, and biomechanical data ( P <0.001). CONCLUSIONS: This comprehensive description of baseline features of KC patients at a tertiary center provides a reference for further longitudinal and international multicentric studies.

Differential Diagnosis of Keratoconus Based on New Technologies.

Keratoconus (KC) must be distinguished from other corneal ectatic diseases and thinning disorders for stage-appropriate and suitable management of each condition. The most relevant corneal pathologies that may imitate the tomographic KC pattern are pellucid marginal degeneration (PMD), keratoglobus, posterior keratoconus, and Fuchs-Terrien marginal degeneration (FTMD). In moderate cases of KC, differentiation is typically possible using slit lamp examination and corneal tomography with evaluation of the location of the corneal thinning region. In early cases, however, differential diagnosis may be more challenging since the cornea may look relatively normal. In severe cases, the extended area of corneal thinning also complicates differentiation. Biomicroscopic findings cannot always give all the information needed to distinguish KC from related ectatic corneal conditions. The aim of this work is to discuss contemporary techniques and findings to assist physicians to identify the correct diagnosis. Corneal topography has been used in recent decades as the main tool for imaging in ectatic corneal diseases. Moreover, Scheimpflug cameras (corneal tomographers), which analyze both anterior and posterior corneal surfaces, curvatures, pachymetry, elevation data, higher order aberrations, Fourier analysis of keratometric data, and corneal density have become the most promising tools for diagnosis and follow-up of ectatic diseases. A noninvasive air pulse tonometer in conjunction with an ultrahigh-speed Scheimpflug camera complements tomographic findings by analyzing biomechanical corneal properties. Α confocal microscopy system, which is a novel clinical technique for the study of corneal cellular structure, could contribute effectively in the same direction. Moreover, anterior segment optical coherence tomography (AS-OCT) creates cross-sections, which can be generated into a three-dimensional structure to produce corneal epithelial thickness (ET) measurements. ET mapping is increasingly recognized as a sensitive tool for the diagnosis of ocular surface disorders. Combining information of all these systems could lead to a more effective identification and differential diagnosis of ectatic corneal disorders.

Impact of Hypothyroidism on Tomography and Biomechanics in Keratoconus - Cross-Sectional and Longitudinal Assessment within the Homburg Keratoconus Center at the Time of Inclusion and after 1 Year.

BACKGROUND AND PURPOSE: The etiology of keratoconus (KC) is probably multifactorial but remains essentially unknown. Previous scientific observations have suggested that hypothyroidism might play a role in the development and progression of KC. The purpose of this study was to analyze the tomographic and biomechanical parameters in KC patients with or without hypothyroidism. METHODS: Twenty-eight patients with KC and hypothyroidism (HT group) and fifty-six KC patients without thyroid dysfunction (WHT group) with matching gender and age were analyzed. Mean age was 40.3 years (range 14 - 57) in the HT group and 40.3 years (range 14 - 57) in the WHT group. Routine ophthalmic examinations consisted of corneal tomography and biomechanical parameters. We extracted the following KC parameters from the Pentacam (Pentacam HR, Oculus, Wetzlar, Germany): Keratoconus Index (KI), maximum keratometry (Kmax), astigmatism, and thinnest pachymetry (TP). From the ocular response analyzer (ORA, Reichert Ophthalmic Instruments, Depew, NY, USA), we extracted corneal hysteresis (CH), corneal resistance factor (CRF), and KC match index (KMI). RESULTS: The comparison of the tomographic and biomechanical values from cross-sectional and longitudinal analyses showed no significant differences between the HT and WHT groups. CONCLUSION: The severity of KC based on tomographical and biomechanical parameters does not seem to depend on the presence of hypothyroidism.

Accelerated Corneal Crosslinking for Treatment of Keratoconus in Children and Adolescents under 18 Years of Age.

PURPOSE: To evaluate the efficacy of accelerated (9 mW/cm2, 10 min) epithelium-off (epi-off) corneal crosslinking (A-CXL) in keratoconus (KC) patients < 18 years of age. PATIENTS AND METHODS: Our retrospective study included 41 eyes (25 male and 5 female patients, mean age 15.3 ± 1.2 years) who underwent A-CXL (9 mW/cm2, 10 min) because of progressive KC or critical KC at first presentation or asymmetrical finding in the partner eye. Outcome measures were best-corrected visual acuity (BCVA) and tomography readings (Pentacam HR, Oculus, Wetzlar, Germany), evaluated 2 years, 1 year, 6 months preoperatively, prior to surgery (pre-CXL) and 6 weeks, 6 months, 1 year, 2 years, > 2 years postoperatively (post-CXL). The demarcation line was assessed by anterior segment optical coherence tomography (SS-1000 and CASIA 2, Tomey, Nagoya, Japan). RESULTS: Total deviation value increased statistically significantly 6 months pre-CXL to pre-CXL. Anterior steep, flat, and mean keratometry values showed a statistically significant increase 6 weeks post-CXL (p < 0.05), followed by a significant decrease until 2 years post-CXL for steep and mean keratometry. Kmax increased 6 weeks post-CXL without statistical significance and decreased significantly even > 2 years post-CXL (p < 0.0001). Posterior keratometry as well as anterior and posterior astigmatism did not show any significant changes post-CXL. Thinnest and apical pachymetry decreased significantly until 6 months post-CXL (p < 0.05) and remained stable > 2 years post-CXL (p > 0.05). BCVA decreased 6 weeks post-CXL but improved significantly > 2 years post-CXL (p = 0.003). The demarcation line reached an average depth of 52.1%. CONCLUSIONS: Epi-off A-CXL stabilizes KC progression in patients < 18 years even > 2 years postoperatively and leads to a deep demarcation line. A "pseudoprogression" is observed up to the 6-week follow-up, which is not indicative of the long-term results.

The Biomechanical E-Staging: In Vivo Biomechanics in Keratoconus. / Das biomechanische E-Staging: In-vivo-Biomechanik beim Keratokonus.

Belin's ABCD keratoconus classification system allows keratoconus staging based on the criteria of anterior (A) and posterior (B) corneal curvature, thinnest corneal thickness (C), and best spectacle-corrected visual acuity (D). These parameters also provide a progression assessment, but do not take corneal biomechanics into account. The analysis of corneal biomechanics by the Corvis ST (Oculus, Wetzlar, Germany) allows for separation of healthy and keratoconus corneas, based on the Corvis Biomechanical Index (CBI) and the Tomographic Biomechanical Index (TBI). As Corvis ST measurements are highly reliable and are independent of keratoconus severity, a biomechanical parameter was developed for keratoconus corneas based on the linear term of the CBI. This provides biomechanical keratoconus staging. The Corvis Biomechanical Factor (CBiF) is the basis for the introduction of the biomechanical E-staging, which augments the ABCD classification to the ABCDE classification, thus including the cornerstone of corneal biomechanics. This article highlights strengths and limitations of the ABCDE classification. "Unilateral keratoconus" supposedly turns out to be mostly a snapshot of a highly asymmetric keratectasia. Regular astigmatism is sometimes an important differential diagnosis to keratectasia and may be difficult to differentiate from it. Furthermore, the use of the biomechanical E-staging in daily practice for progression assessment of keratoconus and after its treatment by corneal cross-linking or implantation of intracorneal ring segments will be demonstrated and discussed.

Risk Factors for Keratoconus Progression in Children Compared with Young and Middle-aged Adults.

BACKGROUND: To investigate the risk factors for keratoconus progression in children (10 - 18 years old; age group 1) compared to young adults (19 - 35 years old; age group 2) and middle-aged adults (36 - 55 years old; age group 3). PATIENTS/METHODS: Ninety-seven children, 445 young adults, and 342 middle-aged adults underwent total ophthalmic examination including clinical refraction, slit lamp examination, corneal tomography, eye biometry, and biomechanical properties measurements. Comparisons were assessed among three age groups and between progressive and nonprogressive eyes. Logistic regression was applied to determine the potential prognostic factors for keratoconus progression in the three age groups. RESULTS: Univariate logistic regression analysis show that the most prominent factors associated with progression were corneal posterior vertical radius (RVP), eye rubbing (RUB), slit lamp corneal thinning (SLT), contact lens use (CL), and central corneal thickness (CCT) in all age groups. Additionally, the anterior chamber volume (ACV) and keratoconus match index (KMI) were associated with progression in age group 1. Location of the thinnest corneal thickness at the vertical axis (TCTy), distance from apex to the thinnest point (BADISTAPEX), scissor reflection in retinoscopy (SKIAREFLEX), and Vogt striae were associated with progression in age group 2, and TCTy, anterior and posterior asphericity (ASPA and ASPP, respectively), BADISTAPEX, SKIAREFLEX, and Vogt striae were associated with progression in age group 3. The multivariate model with the highest predictability indicated RVP, ACV, and SLT as independent determinants of progression in age group 1 (AUC: 90%, sensitivity: 88.9%, specificity: 90.9%), RVP, ACV, SLT, and SKIAREFLEX in group 2 (AUC: 81.6%, sensitivity 88.5%, specificity: 70.3%), and RVP, SLT, Vogt striae, and CL in age group 3 (AUC: 80%, sensitivity 82.8%, specificity: 73%). CONCLUSION: ACV and KMI seem to play a major role in the progression of pediatric KC compared to adults. This is probably due to different anatomical and biomechanical characteristics of a child's eye globe.

Corneal Perforation as a Rare and Late Manifestation of Choroidal Melanoma.

PURPOSE: To report a case of corneal perforation as a rare and late manifestation of choroidal melanoma and to highlight the major histopathological findings of this unusual combined clinical presentation. METHODS: A 74-year-old male patient presented to our department due to corneal perforation of the right eye with the absence of light perception for 6 months. The intraocular pressure was hard on palpation. Because of the protracted finding and reduced visual prognosis, primary enucleation was performed. RESULTS: The histopathological examination revealed choroidal melanoma with epithelioid and spindle cell components at the posterior pole, which was positive for Melan-A, Human Melanoma Black 45 (HMB45), BAP1, and SOX10. The anterior segment showed complete anterior chamber hemorrhage and blood remnants in the trabecular meshwork. The cornea displayed diffuse blood staining with hemosiderin and hemosiderin-loaded macrophages and keratocytes. No inflammatory cells were present near the corneal perforation, which had a width of 3 mm. Intraocular heterotopic ossification was indicative of a long-standing condition. Postoperative cancer staging was normal. CONCLUSION: Corneal perforation should be considered as a very rare and late manifestation of advanced choroidal melanoma and may result from interaction between intraocular hemorrhage, elevated IOP, and its secondary signs such as corneal blood staining.

Approval rates for corneal donation and the origin of donor tissue for transplantation at a university-based tertiary referral center with corneal subspecialization hosting a LIONS Eye Bank.

BACKGROUND: With the increasing demand for corneas, eye banks must optimize the tissue donation, collection, and selection process. This retrospective monocentric study analyzed the approval rates for corneal donation and the origin of and reasons for discarding donor corneas from 2010 to 2019. METHODS: Data included the number of deceased, approval or rejection by the family for corneal donation and contraindications. Corneal grafts were included from all deceased persons who were full-body and multi-organ donors at the Saarland University Medical Center (UKS) and from external institutions. Additional analyzed parameters included endothelial cell count (ECC), blood sample serology for infections, and conjunctival swab testing . RESULTS: A total of 1748 corneoscleral buttons were harvested from 10,265 deceased persons (17% with no contraindication) at the UKS between 2010 and 2019, with a consent rate of 23.3%. The number of keratoplasties increased from 136 in 2010 (15% of the deceased, total = 925) to 251 in 2019 (21%, total = 1214). Both the general and department-specific data showed similar percentages for corneal donation over the years, with intensive care and palliative units recently providing the most corneas. The increase in the number of corneas processed by the cornea bank over the years (368 in 2010 compared with 857 in 2019) was linked both to a better internal supply in 2010 (262, 71.2% of the total) compared with 2019 (519, 60.6%) and to an external supply by reinforcement of cooperation with external hospitals, including Luxembourg in 2010 (106, 28.8% of the total) compared with 2019 (338, 39.4%). A total of 195 of 377 corneas (52%) were discarded in 2009 compared with 260 out of 715 (36%) in 2019. The main reasons for discarding were low ECC (36% of discarded corneas in 2009; 11% in 2019), positive conjunctival swab (11% in 2009; 13% in 2019), and blood sample serology (6% in 2009 and in 2019). CONCLUSION: Despite an increasing number of donors, the demand for corneas is still rising. Improved cooperation with internal departments and with external clinics has led to an increasing number of explanted corneas. The main reason for discarding corneas was low ECC, followed by a positive conjunctival swab for fungal or bacterial contamination and serology. Increased donation rates and continued improvements in collection and selection processes are necessary to cover the high demand for corneas.

Graft Failure after PKP and DMEK: What is the Best Option? / Transplantatversagen nach PKP und DMEK: Was ist die beste Option?

Corneal transplantation is the most commonly performed human tissue transplantation procedure worldwide. Due to the large number of grafts, corneal graft failure has become one of the most common indications for corneal transplantation for immunological and non-immunological reasons (e.g. recurrence of underlying disease, high intraocular pressure, grafted guttae, transmitted HSV or CMV infection). The relatively recently developed lamellar grafting techniques have introduced certain potential complications that may lead to graft failure and require approaches other than penetrating keratoplasty for re-grafting. On the other hand, these new lamellar techniques also offer new possibilities for salvaging failed penetrating grafts, with potential advantages over successive penetrating keratoplasties, such as lower intraoperative risks, faster visual rehabilitation and reduced risk of immune reaction. Today, the patient with good healing who is satisfied with his graft before endothelial decompensation, with low astigmatism and no stromal scars, represents the optimal condition for DMEK after PKP. This can also be combined with phacoemulsification (so-called triple DMEK). Otherwise, a penetrating re-keratoplasty with a larger graft (typically excimer laser repeat PKP 8.5/8.6 mm) is performed to treat edema, scars and irregular astigmatism simultaneously. The medical history carries weight in this decision! Re-DMEK in case of graft failure after DMEK and DSAEK does not require any modification of the standard technique and leads to good visual acuity results if performed quickly. If there is clear stromal scarring after multiple (external) DMEKs, PKP can also be considered to rectify the situation. Otherwise causeless recurrent graft failures must suggest herpetic or CMV endotheliitis and, after PCR analysis of the aqueous humour aspirate, be treated appropriately with medication.

Ocular changes in nephropathic cystinosis: The course of the gold-dust.

PURPOSE: Cystinosis is an autosomal recessive inherited lysosomal storage disease with an incidence of 1:100.000 up to 1:200.000 caused by a gene mutation of a lysosomal transport protein resulting in deposition of cystine in lysosomes in all cells and tissues. In the cornea, crystalline, gold-dust deposition of cystine leads to visual impairment, recurrent erosions, photophobia, epiphora and blepharospasmus. Standard therapy is topical and systemic application of cysteamine which may resolve the accumulated cystine crystals. PATIENT AND METHODS: This is a case report of a thirty-one-year-old patient who already underwent renal transplantation because of nephropathic cystinosis. Visual impairment by cystine crystal deposition was aggravated by a central avascular pannus formation in his right eye. Penetrating keratoplasty was performed in intention to improve the patient's visual acuity and life quality. RESULTS: After penetrating keratoplasty in the right eye, there was only a slight visual improvement. OCT scans of the macula revealed intraretinal cystine crystals and a cystoid macular edema, which was treated with a bevacizumab injection. Transmission electron microscopy of the excised cornea revealed spiky intracorneal inclusions and confocal in vivo microscopy of the left eye allowed detailed visualization of the cystine crystal deposition. CONCLUSIONS: There is a variability of ocular manifestations of nephropathic cystinosis. Ophthalmologists have a central role in the early diagnosis of cystinosis as mostly the first manifestation are cystine crystals in the cornea. Penetrating keratoplasty may be one of the therapeutical options. Nevertheless, the patient has to be informed about the limited prognosis because of the persisting underlying disease.

Keratectasia severity staging and progression assessment based on the biomechanical E-staging.

Until recently, corneal topography has been the gold standard in detecting keratectasia and monitoring its progression. The recently introduced ABCD tomographic keratoconus staging system focuses on anterior ("A") and posterior ("B") radius of curvature, thinnest corneal thickness ("C"), best-corrected visual acuity with spectacles ("D") and is supplemented with the introduction of the biomechanical E-staging (BEST, "E"). The need for biomechanical staging arose from the fact of altered biomechanical characteristics of keratectasia in comparison to healthy corneas. Ectatic corneas usually exhibit a biomechanical weakening and greater deformation than healthy corneas when exposed to a biomechanical stressor such as a standardized air puff indentation as provided by the Corvis ST® (CST, Oculus, Wetzlar, Germany). The BEST is based on the linear term of the Corvis Biomechanical Index (CBI) and provides a biomechanical keratoconus severity staging and progression assessment within the CST software. This review traces the development of the BEST as an addition to the tomographic ABCD staging system and highlights its strengths and limitations when applied in daily practice for the detection, monitoring and progression assessment in keratectasia.

Altered Corneal Biomechanics According to the Biomechanical E-Staging in Pellucid Marginal Degeneration.

PURPOSE: The purpose of this study was to investigate corneal biomechanics in pellucid marginal degeneration (PMD) compared with healthy controls using Corvis ST (Oculus, Germany) by using the new biomechanical E-staging (based on the Corvis Biomechanical Factor, the linearized Corvis Biomechanical Index) together with tomographic parameters. METHODS: Corneal biomechanical and topographic data of 75 eyes of 75 patients with PMD and 75 eyes of 75 age-matched and sex-matched healthy controls were investigated. Topographic parameters (K1, K2, Kmax, central corneal thickness (CCT), and Belin/Ambrósio Deviation Index (BAD-D) were evaluated in dependence of and correlated with the biomechanically defined E-stages. Biomechanical parameters were also recorded for the 2 groups. RESULTS: Patients with PMD showed higher K2, Kmax, BAD-D, and Corvis Biomechanical Factor values and a lower CCT compared with healthy controls (P < 0.001). The E-stage was positively correlated with K1, K2, Kmax, BAD-D, and intraocular pressure difference and negatively correlated with CCT. Stage-dependent analysis revealed a significant increase in K1, K2, Kmax (P < 0.001), and BAD-D (P = 0.041) in stage E3 compared with E0 and a significant decrease in stage E2 in CCT (P = 0.009) compared with E0. CONCLUSIONS: This study showed that patients with PMD may have a reduced corneal stiffness compared with healthy controls which worsens with increasing E-stage. Significant changes in topographic parameters were observed at stage E2 for CCT and at stage E3 for K1, K2, Kmax, and BAD-D when compared with stage E0.

Evaluation of dynamic corneal response parameters and the biomechanical E-staging after Intacs® SK implantation in keratoconus.

PURPOSE: This retrospective longitudinal study evaluated the biomechanical E-staging in KC corneas before and after intracorneal ring segment (ICRS) implantation (Intacs® SK, Addition Technology, Illinois, United States). METHODS: Biomechanical E-staging for ectatic corneal diseases was applied retrospectively on 49 KC corneas of 41 patients who underwent ICRS implantation. The main outcome parameters included the Corvis Biomechanical Factor (CBiF, the linearized Corvis Biomechanical Index and the biomechanical parameters included), the resulting biomechanical E-staging, the stress-strain index, thinnest corneal thickness (TCT), maximal anterior keratometry (Kmax), and the anterior radius of curvature (ARC). They were evaluated at 1.9 ± 1.1 months preoperatively and postoperatively after 2.8 ± 0.7, 5.8 ± 1.0, and 10.6 ± 2.3 months. RESULTS: The CBiF decreased (4.9 ± 0.5 | 4.7 ± 0.5, P = 0.0013), and the E-staging increased significantly (2.8 ± 0.8 | 3.1 ± 0.9, P = 0.0012, paired t-test) from preoperatively to the first postoperative follow-up. The difference remained significant after 6 months; however, there was no more difference after 11 months. TCT was stable, whereas Kmax and ARC significantly decreased after ICRS implantation (TCT: 464 ± 49, 470 ± 51, 467 ± 38, 461 ± 48; Kmax: 56.3 ± 4.5, 54.7 ± 4.5, 54.2 ± 4.8, 54.1 ± 4.3; ARC: 51.5 ± 3.4, 48.3 ± 3.8, 48.6 ± 3.0, 48.6 ± 3.2 preoperatively and 3, 6, and 11 months postoperatively, respectively). Besides Kmax and ARC, Ambrósio's relational thickness to the horizontal profile (ARTh) was the only parameter that was significantly lower than preoperatively at any follow-up (P ≤ 0.0024, Wilcoxon matched-pairs test). CONCLUSION: Intacs® SK implantation results in an increasing biomechanical E-staging in the first postoperative months with stabilization near preoperative values after 1 year. Significantly lower ARTh values at any follow-up document the ICRS effect and contribute to a slightly higher postoperative biomechanical E-staging value.